Retinoblastoma medical therapy
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Simrat Sarai, M.D. [2]
Overview
The optimal therapy for retinoblastoma depends on several factors such as tumor size, tumor location, presence or absence of vitreous or subretinal seeds, and patient age. The various treatment modalities for retinoblastoma include enucleation, external beam radiation therapy, radioactive plaques (I-125 brachytherapy), cryotherapy, laser photoablation, thermotherapy, and chemotherapy (which includes systemic, intra-arterial, and [[intravitreal.[1]
Medical therapy for intraocular tumors
The priority of retinoblastoma treatment is to preserve the life of the child, then to preserve vision, and then to minimize complications or side effects of treatment. The exact course of treatment will depend on the individual case and will be decided by the ophthalmologist in discussion with the paediatric oncologist. Children with involvement of both eyes at diagnosis usually require multimodality therapy (chemotherapy and local therapies).[1] The goals of therapy are the following:[2]
- Eradicate the disease to save the patient's life
- Preserve as much vision as possible
- Decrease risk of late sequelae from treatment, particularly subsequent neoplasms
The choice of therapy depends upon several factors such as:[3]
The various treatment modalities for retinoblastoma include:
Enucleation of the eye
Most patients with unilateral disease present with advanced intraocular disease and therefore usually undergo enucleation, which results in a cure rate of 95%. In bilateral retinoblastoma, enucleation is usually reserved for eyes that have failed all known effective therapies or without useful vision.
External beam radiotherapy (EBR)
The most common indication for EBR is for the eye in a young child with bilateral retinoblastoma who has active or recurrent disease after completion of chemotherapy and local therapies. However, patients with hereditary disease who received EBR therapy are reported to have a 35% risk of second cancers.
Brachytherapy
Brachytherapy involves the placement of a radioactive implant (plaque), usually on the sclera adjacent to the base of a tumor. It used as the primary treatment or, more frequently, in patients with small tumors or in those who had failed initial therapy including previous EBR therapy.
Thermotherapy
Thermotherapy involves the application of heat directly to the tumor, usually in the form of infrared radiation. It is also used for small tumors
Laser photocoagulation
Laser photocoagulation is recommended only for small posterior tumors. An argon or diode laser or a xenon arc is used to coagulate all the blood supply to the tumor.
Cryotherapy
Cryotherapy induces damage to the vascular endothelium with secondary thrombosis and infarction of the tumor tissue by rapidly freezing it. Cryotherapy may be used as primary therapy for small peripheral tumors or for small recurrent tumors previously treated with other methods.
Systemic chemotherapy
Systemic chemotherapy has become forefront of treatment in the past decade, in the search of globe preserving measures and to avoid the adverse effects of EBR therapy. The common indications for chemotherapy for intraocular retinoblastoma include tumors that are large and that cannot be treated with local therapies alone in children with bilateral tumors. It is also used in patients with unilateral disease when the tumors are small but cannot be controlled with local therapies alone. In children with retinoblastoma that has invaded into the choroid, optic nerve, sclera, orbit or anterior chamber require chemotherapy as they are at greatest risk for metastasis and death. Those children without evidence of invasion do not require chemotherapy.
Intra-arterial chemotherapy
Chemotherapeutic drugs are administered locally via a thin catheter threaded through the groin, through the aorta and the neck, directly into the optic vessels.
Nano-particulate chemotherapy
To reduce the adverse effects of systemic therapy, subconjuctival (local) injection of nanoparticle carriers containing chemotherapeutic agents (carboplatin) has been developed which has shown promising results in the treatment of retinoblastoma in animal models without adverse effects
| Patient Group | Treatment Line | Treatment |
|---|---|---|
| With vitreous seeding | ||
| With gross anterior chamber involvement or neovascular glaucoma or orbital inflammation | First | Enucleation |
| Adjunct | Postoperative systemic chemotherapy | |
| Without gross anterior chamber involvement or neovascular glaucoma or orbital inlammation | First | Systemic or intra-arterial chemotherapy |
| Plus | Concurrent laser ablation or chemotherapy | |
| Second | External beam radiation therapy | |
| Third | Periocular carboplatin therapy | |
| Fourth | Enucleation | |
| Without vitreous seeding | ||
| Tumor >2 disc diameters in size | First | Systemic or intra-arterial chemotherapy |
| Plus | Concurrent laser ablation or cryotherapy | |
| Second | External beam radiation therapy | |
| Third | Periocular carboplatin therapy | |
| Fourth | Enucleation | |
| Tumor 2 disc diameters or less in size | First | Focal laser ablation alone |
| Second | Systemic or intra-arterial chemotherapy | |
| Plus | Concurrent laser ablation or cryotherapy | |
| Third | External beam radiation therapy | |
| Fourth | Periocular carboplatin therapy | |
| Fifth | Enucleation | |
| Metastatic disease | First | Multimodal therapy |
| Recurrence | ||
| Post globe-salvaging therapy | First | Brachytherapy |
| Post enucleation | First | External beam radiation therapy |
| Adjunct | Systemic chemotherapy | |
References
- ↑ 1.0 1.1 Retinoblastoma. Wikipedia(2015) https://en.wikipedia.org/wiki/Retinoblastoma Accessed on October 10 2015
- ↑ Retinoblastoma Treatment. National Cancer Institute(2015) http://www.cancer.gov/types/retinoblastoma/hp/retinoblastoma-treatment-pdq#section/_53 Accessed on October 13 2015
- ↑ Shields CL, Shields JA (1999). "Recent developments in the management of retinoblastoma". J Pediatr Ophthalmol Strabismus. 36 (1): 8–18, quiz 35-6. PMID 9972509.